儿童伯基特淋巴瘤186例临床特征及疗效分析

Clinical and prognostic analysis of 186 children with Burkitt＇s lymphoma

目的 分析儿童伯基特淋巴瘤的临床特点及预后相关因素,总结北京儿童医院成熟B细胞淋巴瘤方案的疗效.方法 回顾性病例研究.收集北京儿童医院2007年1月至2015年12月收治的186例年龄≤18岁的初治伯基特淋巴瘤病例,剔除3例因放弃治疗出组病例后,183例按照北京儿童医院高剂量短疗程的改良法国儿童肿瘤协会系列研究（LMB）89方案分层治疗（危险度A组1例,B组59例,C组123例）,C组中97例联合应用利妥昔单抗.分析患儿临床特点、治疗效果,采用Kaplan-Meier方法进行生存分析,COX回归筛选影响预后因素.结果 186例患儿发病时中位年龄5（1 - 14）岁,男159例（85.5％）,女27例（14.5％）,男女比例5.9∶1,174例患儿（93.5％）临床分期已达Ⅲ-Ⅳ期.8例未缓解并进展至死亡,9例复发,治疗相关病死率2.7％（5/183）.中位随访时间48.0（0.5- 128.0）个月,5年总生存率（0S）（89.1±2.3）％、5年无事件生存率（EFS）（87.8±2.5）％.危险度B组和C组5年EFS分别为（94.9±2.9）％、（84.0±3.4）％,组间比较差异有统计学意义（x2=4.258,P=0.039）.危险度C组患儿中单纯化疗组、利妥昔单抗联合化疗组5年EFS分别为（73.1±8.7）％、（86.7±3.7）％,差异无统计学意义（x2=3.360,P=0.067）.中枢神经系统侵犯、早期化疗不敏感、中期评估有瘤灶为预后不良因素（HR=6.167、9.102、3.104,95％CI：2.293- 16.592、1.837-45.107、1.182- 8.153）.结论 高剂量短疗程的改良LMB 89方案对儿童伯基特淋巴瘤疗效显著,联合利妥昔单抗可以使疗效提高,中枢神经系统侵犯、早期化疗不敏感、中期评估有瘤灶与预后不良相关.

Objective To analyze the clinical features and prognostic factors of childhood Burkitt＇s lymphoma and to summarize the therapeutic effect of the mature B-cell lymphoma regimen of Beijing Children＇s Hospital.Methods It was a retrospective study.From January 2007 to December 2015,186 patients below 18 years of age with newly diagnosed,untreated Burkitt＇s lymphoma were enrolled.Three cases were eliminated because of the abandonment of the treatment and 183 cases were stratified and treated according to the mature B-cell lymphoma regimen of Beijing Children＇s Hospital,groups were as follows：A,n =1;B,n=59;C,n=123 and 97 patients in group C received combined rituximab therapy during the treatment.The clinical features and therapeutic effects of patients were analyzed,overall survival （OS） and event-free survival （EFS） were estimated by the Kaplan-Meier method.COX regression was used to identify the prognostic factors.Results The median age at diagnosis was 5 （1-14） years.There were 159 males （85.5%） and 27 females （14.5%）,the male-to-female ratio was 5.9∶ 1.A total of 174 cases （93.5%） evolved to stage Ⅲ and Ⅳ.Eight cases did not achieve remission and progressed to death,9 cases relapsed.Only 5 patients （2.7%） died of treatment-related complications.With a median follow-up time of 48.0 （0.5-128.0） months,the 5-year OS rate and EFS rate were （89.1 ±2.3）% and （87.8 ±2.5）%.There was significant difference in the 5-year EFS rate between group B and C （（94.9±2.9）% vs.（84.0±3.4）%,x2=4.258,P=0.039）.The 5-year EFS rate was （73.1 ±8.7）% and（86.7±3.7）% for patients in the group C treated with chemotherapy only and those treated with chemotherapy combined rituximab,but no statistical difference was found between them （x2=3.360,P=0.067）.Central nervous system （CNS） involvement,insensitivity to early phase chemotherapy,residual diseases in mid-term evaluation were independent unfavorable prognostic factors （HR=6.167,9.102,3.104,95%CI：2.293-16.592,1.837-45.107,1.182-8.153）.Conclusions The large dose,short course treatment of mature B-cell lymphoma regimen of Beijing Children＇s Hospital is effective for pediatric Burkitt＇s Lymphoma.Combined treatment with rituximab can improve the efficacy.CNS involvement,insensitivity to early phase chemotherapy,residual diseases in mid-term evaluation are associated with increased risk of poor prognosis.